On the 30th of October, we celebrated Rynah’s 2nd birthday. While it was a day filled with celebrations, the smell of blown out candles and sweet memories – it was bitter-sweet. It was the first birthday with the shadow of CF’s diagnosis in the room. Both filled with joy and sadness on this day, I was reminded that our time on earth is temporary and we should live each day as if it were our last.* This is a bitter pill to swallow, but with the grace of God, I’ve grown to accept it most of the time!
Rynah looked so beautiful in her birthday dress at her party. She loved the attention, acting all shy and pretty. When looking at photos of her, it is almost impossible to think that this perfect little angel has a destructive disease inside her. Hidden from those who are not aware. Both friends and strangers have said, “ah she will outgrow it…” I have to bite my lip and try to explain without sounding like a drama queen – no she won’t, this is not something you ever outgrow, in fact, quite the opposite This is a progressive genetic disorder that worsens with age (medically spoken that is). And with this, I felt it is important to post more information about Cystic Fibrosis.
If I had to define CF briefly, the definition would have to include that you are born with it, that it is progressive and therefore automatically shortens life expectancy. The rest of it would be details.
What is CF
In short – you cannot outgrow CF – in fact, it is known to be progressive. A person with CF is never ‘healthy’ or normal as their breathing takes more effort, their bodies work harder to function. As you grow older your body changes, the damage caused by infections and constant inflammation lead to organ failure – hence the need for lung and other organ transplants – often this means a CF person cannot grow old. (Old being older than 30s/ 40s if you are lucky). We are lucky – a few decades back CF children never reached adulthood, or their teens even. So we are filled with hope that Rynah will indeed grow old, not only medically, but by the miracle of God’s grace. We accept her path and reality though, it is not in our hands. For more details and information please visit the CF Foundation’s Website here.*Important to note here – despite the severity of the disease, its chronic nature and shortened life expectancy, this disease is not acknowledged as a chronic illness in South Africa. This limits our access to medicine out there. We need to be on the highest tier of a medical aid fund to have access to chronic illness benefits. We simply cannot afford this yet – but we trust we will have a plan in place for when she turns 6, as our options at Red Cross will change at this age.The late Claire Wineland explained it so well…
How is it treated
There is currently no cure for Cystic Fibrosis. While there is a lot of medical breakthroughs, treatment mostly involves trying to stop the progression. In South Africa, we do not have access to the latest medicines that slow down the progression. We will have to work on gaining the interest of pharmaceutical companies like Vertex to trial drugs in South Africa. But until then, we have a team that supports us to maintain Rynah’s health as much as possible. We do what we can and trust God for the rest.
Our new normal
While Rynah looks healthy and so far we have been so blessed with her health. But it takes a lot of work and even more grace from God. I don’t like moaning about this part as I’ve come to realise that this is just part of our lives now. As simple as brushing teeth or bathing. But I would like to share what her treatments entail for now – while she is healthy.
She is nebulised with 5% saline. This is very salty stuff – used in hospitals for IV administration – it helps loosen the mucus by adding salt to the lungs pathways so that it can move more easily. As it can irritate the chest, she uses an asthma inhaler before treatment to open her chest. We don’t have nebuliser from the local pharmacy, no thanks to family we have a proper PariJnr nebuliser. It is more powerful than the average nebuliser. It takes about 15 to 20 minutes to nebulise her. Then there is a 20-minute wait (sometimes this is not possible but most days this is routine). Then 10 minutes of percussion physio (cupped hand striking across the back/shoulder in a rhythmic manner), 5 minutes per side. This is part of airway clearance, moving the mucus out of the lungs as much as possible. Then there are breathing exercises (blowing bubbles for example) or just trying to get her to be active.
This is done twice per day and takes up about an hour at a time. In between, I have to make sure her nebuliser parts are cleaned and sterilised properly. While it is only necessary to sterilise once per week, I prefer sterilising after each use.
As her pancreas is insufficient it means she cannot digest food properly. To help her, she gets Creon (enzymes) with each meal or snack. Either half a tablet or one tablet, depending on how much she eats. It comes in a tablet form, but luckily the tablet is filled with granules, and I give this to her with the help of a Squishy so that it doesn’t get stuck in her throat or mouth. This was a challenge initially but has become almost second nature.
And talking about food – because her body works harder than ours due to the sticky mucus, she has to eat almost 3 times more than an average child her age. This is tough! Her diet needs to be high protein and high in fats. She also needs more salt – the root of the problem lies with the genes that instruct the salt channels. She loses most of her salt through her sweat – which means dehydration is a real risk on hot days.
Lastly, she gets a high regimen of vitamins and an immune booster.
When she is sick, the above treatments are intensified to 3 to 4 times per day and could lead to admission in the hospital to keep an eye on her breathing. (We had this in May this year with RSV).
Rynah also goes for standard checkups (currently every two months), at the CF clinic at the Red Cross Hospital. Her sputum is checked for bacterial growth, they listen to her chest, check her weight and length (it is important that she grows and gains well).
Our new world
Our world has changed drastically. We are learning daily…But at the same time, we have grown accustomed to it. We have a little shoe rack and hand sanitiser at our door. To try and limit germs from being treaded into the house. While we are not overly cautious, we are just doing what we can.
It has also meant we cannot send Rynah to school until she is 5. Just so that she builds up an immune system as much as she can. This means I have to work from home – luckily I’ve been doing this all along, but with the new schedule, it can be taxing. We need practical support and have had to ask for help in so many ways – not only financially but also emotionally. This has not been easy. While we have the support of many fellow CF moms – we also cannot meet with each other. Our children cannot mingle as they may cross-infect each other with dangerous bugs.
We have two children and this has been hard on our eldest. Yes, there is the normal jealousy of having a sibling, but our eldest has also had to grow used to Rynah getting a lot more attention than him. With her treatments, it is very difficult to balance. He is also fighting his own battles at school, with a delay in coordination development and struggles with fine and gross motor skills. Highly intelligent, we know he will have the grit to get through his challenges. He is a sensitive boy, but tough at the same time.
But besides all the stresses and strains of the practical adjustment at home, there are the stresses that go with going out. trying to have her live a normal life, not wrap her up in cotton wool, but at the same time being aware that any germ has the potential to infect her lungs. I have to sanitise her hands, give her medicine before she eats, move away from anyone coughing. Also being a toddler, there is so much I have to be aware of. She cannot share cups or utensils with other children, not even her brother, nor can she eat stuff she dropped on the ground. Imagine a group of kids, naturally putting toys in their mouths – I have to make sure she doesn’t do the same. Germs are everywhere, in mud, in the sand in leaves, but especially in standing water.
BUT no matter what we do, we cannot protect her from all germs. So we have to let her live, do what we can and trust God with her life in His hands.
So with this, I hope I’ve shed a bit more light on the basics. I don’t want to dwell on this, but it is so important to create awareness. We all fight our own battles and I just wanted to share a bit of our daily battle. While we falter daily, we are positive and we are so blessed to learn so much from our little fighter. No one person’s path with CF is the same, no one knows what Rynah’s path will look like. Only God does – and we will walk each step of the way with her, to support her and teach her to be who she needs to be in God.
“I praise you because [Rynah is] fearfully and wonderfully made; your works are wonderful, I know that full well.” Psalm 139:14
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